evrazkarЕвразийский Кардиологический ЖурналEurasian heart journal2225-16852305-0748Евразийская ассоциация кардиологов10.38109/2225-1685-2017-4-28-39evrazkar-256Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL PAPERSКЛИНИЧЕСКИЕ ОСОБЕННОСТИ ЛЕГОЧНОЙ АРТЕРИАЛЬНОЙ ГИПЕРТЕНЗИИ, АССОЦИИРОВАННОЙ С ВРОЖДЕННЫМИ СИСТЕМНО-ЛЕГОЧНЫМИ ШУНТАМИCLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTSГрацианскаяС. Е.GratsianskayaS. Ye.

Аспирант отдела легочной гипертензии и заболеваний сердца

121552, г. Москва, 3-я Черепковская, д. 15 а

тел. 8-495-414-68-42

Postgraduate student of the department of pulmonary hypertension and heart disease

121552, Moscow, 3rd Street Cherepkovskaya, 15 a

tel. 8-495-414-68-42

svetik_lap@mail.ruАрхиповаО. А.ArkhipovaO. A.

К.м.н., научный сотрудник отдела легочной гипертензии и заболеваний сердца

121552, г. Москва, 3-я Черепковская, д. 15 а

тел.: 8-495-414-68-33

PhD, Researcher of department of pulmonary hypertension and heart disease

121552, Moscow, 3rd Street Cherepkovskaya, 15 a

tel. 8-495-414-68-33

Olga_ark@list.ruЗоринА. В.ZorinA. V.

К.м.н., научный сотрудник отдела легочной гипертензии и заболеваний сердца

121552, г. Москва, 3-я Черепковская, д. 15 а

тел.: 8-495-414-68-42

PhD, Researcher of the department of pulmonary hypertension and heart disease

121552, Moscow, 3rd Street Cherepkovskaya, 15 a

tel.: 8-495-414-68-42

zorandvla@list.ruМартынюкТ. В.MartynyukT. V.

Д.м.н., руководитель отдела легочной гипертензии и заболеваний сердца

121552, г. Москва, 3-я Черепковская, д. 15 а,

тел.: 8-495-414-64-50

MD, leading researcher, Head of the department of pulmonary hypertension and heart disease 

121552, Moscow, 3rd Street Cherepkovskaya, 15 a,

tel. 8-495-414-64-50

trukhiniv@mail.ruЧазоваИ. Е.ChazovaI. Ye.

Академик РАН, профессор, директор 

121552, г. Москва, 3-я Черепковская, д. 15 а

тел.: 8-495-414-63-05

Academician of Russian Academy of Science, MD, Director 

121552, Moscow, 3rd Street Cherepkovskaya, 15 a

tel. 8-495-414-63-05

noemail@neicon.ruФГБУ «Национальный медицинский исследовательский центр кардиологии» Минздрава РоссииРоссияNational Medical Research Centre of cardiology of Ministry of HealthRussian Federation201730122017042839Copyright © Грацианская С.Е., Архипова О.А., Зорин А.В., Мартынюк Т.В., Чазова И.Е., 20172017Грацианская С.Е., Архипова О.А., Зорин А.В., Мартынюк Т.В., Чазова И.Е.Gratsianskaya S.Y., Arkhipova O.A., Zorin A.V., Martynyuk T.V., Chazova I.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.heartj.asia/jour/article/view/256

Легочная артериальная гипертензия (ЛАГ), ассоциированная с врожденными пороками сердца (ВПС) (ЛАГ-ВПС), несмотря на сходство с точки зрения гистологического поражения легких, заметно отличается от других форм ЛАГ и представляет собой гетерогенную популяцию пациентов с различной локализацией дефектов, сопутствующими заболеваниями, показаниями и противопоказаниями к хирургической коррекции, кроме того часть пациентов имеют в анамнезе оперативное лечение порока. Согласно рекомендациям Европейского Общества Кардиологов по диагностике и лечению ЛГ 2015 года, в клинической классификации выделяют четыре основные группы ЛАГ-ВПС: 1) синдром Эйзенменгера, 2) ЛАГ, ассоциированную с преимущественно системно-легочными шунтами, 3) ЛАГ при малых, случайных дефектах, 4) ЛАГ после хирургической коррекции пороков. За последние годы отмечается эволюция рекомендаций по коррекции ВПС. Стратификации риска имеет большое значение для уточнения прогноза развития болезни, ответа на терапию и сроков хирургической коррекции. Для определения тактики оптимального лечения требуется тщательный и экспертный подход к диагностике и дифференциальной диагностике. Изучение клинических особенностей, стратификации риска и оценки влияния генетического фона помогут определить наилучшие терапевтические и тактические подходы, что в свою очередь связано с улучшением качества жизни и повышением выживаемости пациентов с ЛАГ-ВПС.

Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD) (PAH-CHD), despite the similarities of histological lesions, is а different from other forms of PAH. PAH-CHD is represented by a heterogeneous patient population with varying defect locations, concomitant diseases, indications and contraindications to surgical correction; also, some patients have a history of a defect closure. According to the European guidelines for the diagnosis and treatment of pulmonary hypertension 2015, there are four main groups in the clinical classification of PAH-CHD: 1) Eisenmenger’s syndrome, 2) PAH associated with prevalent systemic-to-pulmonary shunts, 3) PAH with small/concidental defects, 4) PAH after defects correction. The evolution of the guidelines for the surgical correction of CHD has seen in recent years. Risk stratification has a great importance for further prognosis, response to treatment and timing of surgical correction. For optimal treatment carefulness and expertise in diagnosis and differential diagnosis should be exercised. A better understanding of clinical features, risk stratification and the assessment of the impact of a genetic background will help to determine the best clinical management, which is associated with better quality of life and improved survival in patients with PAH-CHD.

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The authors declare that there are no conflicts of interest present.